The association between external auditory canal osteoma and external auditory canal cholesteatoma is rare, with only a few reports in the current literature. Intracranial complications are very rare in the external auditory canal cholesteatoma, especially with direct propagation through the internal auditory canal. A case of 27-year-old male presenting with external auditory canal osteoma with secondary external auditory canal cholesteatoma is described. Progression of the disease created in turn a mastoiditis, labyrinthitis and pachymeningitis confined to the internal auditory canal. The patient was treated by a subtotal petrosectomy, without entering the internal auditory canal. A control MRI after 3 months showed reduction of the internal auditory canal enhancement.